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First Case of Canine Pulmonary Non-Langerhans Cell Histiocytosis
Frontiers in Veterinary Science 2025
Pulmonary non-Langerhans cell histiocytosis in a dog with acute interstitial granulomatous pneumonia
Granger KL Jr, Williams K, Ohlendorf A, Shropshire S, Hall KE
Background: Pulmonary non-Langerhans cell histiocytosis (PNLCH) is a rare histiocytic disorder in humans, classified as Erdheim-Chester disease or Rosai-Dorfman disease. In veterinary medicine, a similar pulmonary histiocytic disease has not been reported in dogs. This case describes an 8-year-old male Rottweiler with acute, progressive respiratory failure, ultimately diagnosed post-mortem with PNLCH.
Methods: The patient presented with worsening respiratory distress and lethargy. Diagnostic imaging showed multifocal alveolar patterns with nodular lesions. Despite empirical treatments, clinical deterioration led to euthanasia. Post-mortem examination included histopathology, special stains, and immunohistochemistry, confirming a histiocytic infiltrative process consistent with PNLCH.
Results: The dog exhibited severe histiocytic infiltration in the lungs without evidence of infectious agents. Immunohistochemical findings were consistent with non-Langerhans histiocytic disease, with markers such as CD204 and IBA-1 confirming a macrophage origin. The case also showed similarities to acute interstitial pneumonia (AIP) in humans.
Limitations: The study is limited by its single case report nature and the lack of established diagnostic criteria for canine PNLCH. Further research is needed to define the condition’s pathophysiology and potential treatment strategies.
Conclusions: This report presents the first documented case of canine PNLCH, broadening the understanding of histiocytic disorders in veterinary medicine. Given the rapid disease progression and poor response to treatment, early recognition and further investigation into potential therapies are critical.

Three-view thoracic radiographs of an 8-year-old Rottweiler with pulmonary non-Langerhans cell histiocytosis. Note the numerous, poorly marginated, variably sized patchy regions of alveolar patterns throughout the pulmonary periphery, some of which have a nodular appearance. L, left; R, right.
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